Multiple System Atrophy -

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Résumé :
This textbook provides a comprehensive monography on multiple system atrophy (MSA), a rare and fatal neurodegenerative disorder that presents with autonomic failure and either parkinsonism (MSA-P) or cerebellar ataxia (MSA-C).The underlying neuropathology of MSA is characterized by striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA) and unique oligodendroglial cytoplasmic alpha-synuclein inclusions. MSA is therefore classified among the alpha-synucleinopathies such as Parkinson`s disease, pure autonomic failure and dementia with Lewy bodies. Over the last decade there have been important advances in early diagnosis, pathogenesis research and clinical trial activity. The monography will cover the entire spectrum ranging from molecular and genetic work to symptomatic and interventional therapies. The book is written for movement disorder clinicians and basic neuroscientists interested in degenerative movement disorders.

Biographie:
Professor Gregor K. Wenning graduated from M?nster University in 1991. He trained in Neurology at the University Hospitals of T?bingen, London and Innsbruck. He started his scientific career at the Institute of Neurology in London where he performed a series of clinicopathological and experimental studies on multiple system atrophy (MSA). He received a PhD from the University of London in 1996. Since 1999 he has served as Consultant Neurologist and Assistant Professor at the Parkinson Centre of the Department of Neurology at the Medical University Innsbruck. In 2006 he became a full Professor at the Medical University of Innsbruck and head of the newly created Division of Clinical Neurobiology devoted exclusivley to MSA research. Together with Professor Werner Poewe he initiated the European MSA Study Group in 1999. EMSA-SG generated the first validated assessment tool for MSA (Unified MSA Rating Scale - UMSARS), it also conducted a large natural history study in 12 European countries and Israel, and currently performs genomic studies as well as phase II intervention trials. Professor Wenning has published more than 200 original papers, reviews and invited book chapters on MSA and related parkinsonian disorders. He was a member of the German Scholarship Foundation, fellow of the UK Parkinson`s Disease Society, first recipient of the Birkmayer prize in 1998 and also first recipient of the Oppenheimer prize in 2004 for recognition of his work on MSA.Dr. Alessandra Fanciulli graduated with distinction from the Sapienza University of Rome in 2010. She started her scientific career in Rome working on cardiovascular autonomic failure in neurodegenerative diseases. Since 2012 she has been a clinical research fellow at the Division of Neurobiology, Innsbruck Medical University and currently performs clinical research projects on multiple system atrophy and Parkinson's disease. In 2013 Dr. Fanciulli was awarded with the research prizes of the LIMPE - Lega italiana per la lotta contro la malattia di Parkinson and ?PG - ?sterreichische Parkinson Gesellschaft.

Sommaire:

1. Historical Review.- 2. Epidemiology.- 3. Neuropathology.- 4. Etiopathogenesis.- 5. Animal Models.- 6. Clinical Presentation.- 7. Clinical diagnostic Criteria.- 8. Natural History.- 9. Investigations.- 10. Tratment.